GI bleeding in an 11 mont...

An 11-month-old boy, weight 8 kg, length of 67 cm, was brought by his mother to the emergency room because of vomiting blackish red blood since 1 day ago with a frequency of 3x a day. There was no black stool, no fever. The patient had complaints of yellowy eyes, face, and body since the age of 3 weeks, defecation like putty. Since the age of 4 months, the mother complained that the patient's stomach was getting bigger. Physical examination found the child looked lethargic, HR 130x/min, palpable weakness, capillary filling time 3 seconds. The skin of the face and whole body appeared icteric. Hepar was not palpable, spleen palpable in Schuffner III-IV.

The supporting examination was obtained:

Hb: 5.5 g/dL Ht: 17% Total bilirubin/directed/indirected: 17,1/15,0/2,1 SGOT: 69 U/L SGPT: 73 U/L

Emergency management :

Hospitalization

- Adequate oxygenation, give oxygen 2 liters/minute

- Nutrition: NPO (temporary fasting), parenteral nutrition

- Install IV line, install NGT drain, install urine catheter

- Loading with lactated ginger 20 mL/kg, target diuresis after fluid resuscitation > 1 ml / kg / hour

- Gastric lavage

- Transfuse PRC until target Hb 8-9 g/dL, monitor transfusion reaction

- Give PPI (omeprazole) IV 1 mg/kg/day every 12-24 hours

- If bleeding persists, give somatostatin analog (Octreotide®) bolus 1 mcg/kg, followed by drip 1-4 mcg/kg/hour

- Endoscopic ligation or sclerotherapy

- Start priming if there is no bleeding

- Broad spectrum antibiotics (cefotaxime) long-term management

- Adequate nutrition with MCT-enriched formula (Pregestimil®) according to RDA when there is no bleeding

- Manage portal hypertension: propranolol 0.5-1 mg/kg/day divided into 3 doses

- Ursodeoxycholic acid 10-30 mg/kg/day divided into 3 doses

- Vitamin A, D, E, K supplementation

- Albumin transfusion if indicated

- Liver transplantation